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Cor pulmonale.

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Chronic Disease↗

Prospective echocardiographic analysis of progressive obstruction of the proximal pulmonary artery in congenital heart disease and obstructed pulmonary flow.

BACKGROUND: It is uncertain whether proximal pulmonary artery (PA) obstruction exists soon after birth and whether its progress relates directly to postnatal ductal constriction in congenital heart disease and obstructed pulmonary flow. METHODS: Serial morphometric analyses of the PA branches by echocardiogram were performed in 28 patients (mean age at initial study 2.5 days) until severe constriction of the ductus occurred (mean age 47 days). These patients were divided into 2 groups by subsequent angiographic or postmortem confirmation; 10 with proximal PA obstruction (group 1) and 18 without obstruction (group 2). RESULTS: At the time of initial examination, the mean indexed diameter of the proximal PA on the side of the ductus arteriosus in group 1 was significantly smaller than that on the contralateral side (5.2+/-0.7 versus 9.0+/-0.7 mm/BSA0.5, P < .001) or that in group 2 (8.0+/-0.4 mm/BSA0.5, P < .001). In group 1, 8 patients had a proximal PA index on the ductal side < or = 5.5 mm/BSA0.5, which was less than those of any group 2 patients. After severe constriction of the ductus, the proximal PA index on the ductal side further decreased only in group 1 (P < .01). CONCLUSIONS: These data indicate that unilateral obstructive lesion of branch PA is present shortly after birth and its progression relates directly to ductal constriction. Neonates with branch PA obstruction can be identified on their initial echocardiogram as having a proximal PA index on the ductal side < or = 5.5 mm/BSA0.5.

Arterial Occlusive Diseases↗

[Hemodynamic effects of prostaglandin E1 in patients with congenital heart disease and pulmonary hypertension].

Investigations were performed to determine whether prostaglandin E1 (PGE1) would get a favorable change in pulmonary hymodynamics in patients with congenital heart disease and elevated pulmonary vascular resistance. Thirty-seven patients were studied. PGE1 was infused into the pulmonary artery with 100-200 ng/kg.min. PGE1 caused a significant decrease in pulmonary artery pressure and in pulmonary resistance in low-resistance group (TPRO < or = 600 kPa.s/L) (P < 0.05). But PGE1 resulted in systemic hypotention in high-resistance group for the same dose (TPRO > 600 kPa.s/L). This study shows that PGE1 is an effective vasodilator. The doses and the effects of PGE1 are dependent on the degree of pulmonary vascular disease.

Adolescent↗

[Systemic-pulmonary shunts in congenital heart diseases with decreased pulmonary blood flow: critical analysis of the surgical techniques and immediate results].

PURPOSE: To evaluate immediate postoperative results in children with congenital heart disease and decreased pulmonary blood flow who underwent a systemic to pulmonary shunt. PATIENTS AND METHODS: Sixty-four patients underwent surgery, 46.8% (30) of them males with ages from 1 day to 17 years old. They were divided in three groups: I--13 pts (20.3%) who underwent classical Blalock-Taussig (BT) shunt; II--46 pts (71.8%) who underwent modified BT shunts, 34 of them with polytetrafluoroetylene (PTFE) and 12 of them with umbilical vein shunts; III--5 pts (7.8%) with central anastomosis that were made with three different types of graft; 3 PTFE, umbilical vein and bovine mammary artery in one each. The simultaneous procedures were: section and suture of PDA--2, closure of systemic-pulmonary collaterals--3, pulmonary valvotomy--3, right ventricular outflow patch--3, pulmonary branch stenosis (enlargement)--2. RESULTS: In group I there were 4 (30.7%) closures, with two immediate reoperation and a total mortality of 30.7%. In group II there were 4 closures (8.6%) and two reoperations with a mortality of 15.2% (7 cases). In group III there was one graft closure and an overall mortality of 80% (4 pts). When analysing deaths due to the shunt itself the mortality rate was respectively 15.3%, 8.6% and 40.0%. CONCLUSION: Modified BT operation was performed most frequently in our service; it was associated with less closure and mortality than the other types of shunt. It is considered our first alternative for a systemic-pulmonary shunt.

Adolescent↗

Emphysema heart.

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Emphysema↗